Ocular Manifestations of Sickle Cell Disease: Signs, Symptoms and Complications

Ophthalmic Epidemiol. 2020 Aug;27(4):259-264. doi: 10.1080/09286586.2020.1723114. Epub 2020 Feb 3.


Background: Sickle cell disease is an inherited hematological disorder that can affect any organ in the body including the eyes (1-6). Previous studies on ocular manifestations of sickle cell disease generally included samples of less than 100 patients. In this study, we aim to assess the frequency of different ocular signs, symptoms and complications among sickle cell disease patients.

Methods: This study was conducted using data from the Cooperative Study of Sickle Cell Disease (CSSCD). Patients with major sickle cell hemoglobinopathies (SS, SC, S β-thal) were eligible for enrollment. Patients from all age groups were included. Patients underwent detailed ophthalmological examination under standardized conditions.

Results: A total of 1904 patients were included in this study, with a mean age of 27.67 (±11.72) years. 1,802 (96.4%) patients had BCVA of more than 20/40 in the better-seeing eye. On slit lamp examination, the presence of vascular loops and segment, representing a positive conjunctival sign, was the most common reported abnormal finding (54.1%). The most common complication was peripheral retinal artery occlusion detected in225 patients (20.3%) bilaterally and 77 patients (6.9%) unilaterally.

Conclusion: In this study that included one of the largest samples ever studied to assess ocular complications of sickle cell disease, we identified the frequency and percentages of different ocular signs, symptoms and complications in different age groups.

Keywords: Sickle Cell; conjunctival sign; ocular; retinopathy; sunburst.

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / diagnosis
  • Anemia, Sickle Cell / ethnology
  • Black or African American / ethnology
  • Case-Control Studies
  • Conjunctiva / abnormalities
  • Conjunctiva / diagnostic imaging
  • Diagnostic Techniques, Ophthalmological / standards
  • Eye Diseases / diagnosis
  • Eye Diseases / etiology*
  • Female
  • Humans
  • Male
  • Prospective Studies
  • Retinal Artery Occlusion / epidemiology
  • Retinal Diseases / diagnosis
  • Retinal Diseases / epidemiology
  • Retinal Diseases / etiology*
  • Vision, Ocular / physiology*
  • Visual Acuity / physiology
  • Young Adult